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Target Concepts:
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Query: UMLS:C0019621 (
Langerhans cell histiocytosis
)
3,250
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Langerhans' cell histiocytosis
(
LCH
) is an abnormal accumulation of dendritic histiocytes of unknown pathogenesis. It has recently been shown to be a clonal process. Bcl-2 is a proto-oncogene whose protein product is known to inhibit apoptosis. The overexpression of
bcl-2
has been demonstrated in a number of neoplasms, presumably prolonging the survival of the neoplastic cells. We examined the expression of
bcl-2
in normal Langerhans' cells in the skin and in
LCH
by immunohistochemistry for protein and in situ hybridization for mRNA to see if it could be implicated in the pathogenesis of this disorder. Additionally, we performed Southern analysis to determine if genomic rearrangement of the
bcl-2
gene occurs in cases of
LCH
. Bcl-2 was not detected in normal skin Langerhans' cells. Eleven of thirteen cases of
LCH
demonstrated
bcl-2
protein expression in the cytoplasm of the Langerhans' cells by immunohistochemistry, while 12 of 13 cases had evidence of
bcl-2
mRNA by in situ hybridization. Southern analysis revealed a germline configuration of the
bcl-2
gene in the five cases studied. These findings suggest that
bcl-2
expression is present and up-regulated in pathologic Langerhans' cells, however, this overexpression does not appear to be due to genomic rearrangement.
...
PMID:Bcl-2 expression in Langerhans' cell histiocytosis. 1046 80
Langerhans cell histiocytosis
(
LCH
) is a clonal neoplasm that shows diverse clinical manifestations and courses of disease progression. The etiology and pathophysiology of
LCH
remain uncertain. We describe the clinical course of a 23-year-old Japanese woman with multi-system
LCH
, who showed rapid progression after steroid reduction and developed multi-organ failure. Liver biopsy showed
LCH
infiltration with fatty degeneration. She was treated with cytarabine, vincristine, and prednisolone according to the Japan
LCH
study group 02 protocol, without any clinical improvement. Low expression of Ki67 and
bcl-2
failed to explain the rapid clinical course. Panhypopituitarism and hypothalamic dysfunction may have caused nonalcoholic fatty liver disease and liver failure. This case indicates that some multi-system
LCH
patients with hypopituitarism and hypothalamic dysfunction show very rapid progression and are difficult to treat.
...
PMID:Rapidly progressing fatal adult multi-organ Langerhans cell histiocytosis complicated with fatty liver disease. 2303 28
